Bottom Line:
Three minor postoperative complications were encountered; a long-term pulmonary complication was seen in one case after reintervention and was successfully resolved after 10 days.Surgery was successful in the other 5 cases, where solid arthrodesis was achieved and no significant curve progression was observed at follow-up.In case of mild spinal deformities in arthrogryposis, brace treatment should be attempted, the evolution of the curves being unpredictable; however, when the curve exceeds 40° and presents with marked hyperkyphosis, hyperlordosis or pelvic obliquity, surgery should not be delayed.

Background: The reported incidence of scoliosis in arthrogryposis varies from 30% to 67% and, in most cases, the curves progress rapidly and become stiff from early age.The authors report six cases of scoliosis in arthrogryposis to assess the role of surgical treatment.

Methods: Six cases (3 males, 3 females; mean age at surgery 13.2 years) with arthrogryposis multiplex congenita associated with the characteristic amyoplasia were reviewed: they were operated on for scoliosis at the authors' Spine Surgery Department between 1987 and 2008.Surgery was performed using the Harrington-Luque instrumentation (2 cases), the Luque system (1), a hybrid segmental technique with hooks and screws (1) and spinal anchoring with pedicle screws (2).

Results: The patients were clinically and radiologically reviewed at a mean follow-up of 4.2 years, ± 2.7 (range, 1 to 9 years). Three minor postoperative complications were encountered; a long-term pulmonary complication was seen in one case after reintervention and was successfully resolved after 10 days. Surgery was successful in the other 5 cases, where solid arthrodesis was achieved and no significant curve progression was observed at follow-up.

Conclusions: The experience acquired with the present case series leads the authors to assert that prompt action should be taken when treating such aggressive forms of scoliosis. In case of mild spinal deformities in arthrogryposis, brace treatment should be attempted, the evolution of the curves being unpredictable; however, when the curve exceeds 40° and presents with marked hyperkyphosis, hyperlordosis or pelvic obliquity, surgery should not be delayed.

Mentions:
Case 2 (female, born in 1976, patient with congenital arthrogryposis). Three months after her birth, x-ray examination of the spine had shown a very mild right convex thoracolumbar scoliotic deviation, which had developed into a curve of 27° with pelvic obliquity of 9° by the age of 6. Full-time brace treatment since the age of 6 had failed to control the progression of the deformity, since the following radiographic values were observed at the age of 15: a stiff structured 111° thoracolumbar scoliosis, 56° lumbar lordosis, 47° thoracic kyphosis, pelvic obliquity of 28° and a grade 3 skeletal maturity according to Risser (Figure 3A and 3B). By the age of 9, she had already undergone surgery twice for bilateral clubfoot congenital deformity and once for bilateral elbow tenolysis. Preoperatively BMI was 15, a severe respiratory deficit was observed and the patient could walk to a limited extent outdoors. Spinal arthrodesis with Luque instrumentation from T2 to L4 was performed at the age of 15, in 1991. Postoperatively, the Cobb angle was 84°, lumbar lordosis 37°, thoracic kyphosis 19° and pelvic obliquity decreased to 19°. A cast orthosis was then applied for 6 months, followed by a CLB brace for 2 years. At a 6-year follow-up, correction was stable and no complications were seen (Figure 4A and 4B).

Mentions:
Case 2 (female, born in 1976, patient with congenital arthrogryposis). Three months after her birth, x-ray examination of the spine had shown a very mild right convex thoracolumbar scoliotic deviation, which had developed into a curve of 27° with pelvic obliquity of 9° by the age of 6. Full-time brace treatment since the age of 6 had failed to control the progression of the deformity, since the following radiographic values were observed at the age of 15: a stiff structured 111° thoracolumbar scoliosis, 56° lumbar lordosis, 47° thoracic kyphosis, pelvic obliquity of 28° and a grade 3 skeletal maturity according to Risser (Figure 3A and 3B). By the age of 9, she had already undergone surgery twice for bilateral clubfoot congenital deformity and once for bilateral elbow tenolysis. Preoperatively BMI was 15, a severe respiratory deficit was observed and the patient could walk to a limited extent outdoors. Spinal arthrodesis with Luque instrumentation from T2 to L4 was performed at the age of 15, in 1991. Postoperatively, the Cobb angle was 84°, lumbar lordosis 37°, thoracic kyphosis 19° and pelvic obliquity decreased to 19°. A cast orthosis was then applied for 6 months, followed by a CLB brace for 2 years. At a 6-year follow-up, correction was stable and no complications were seen (Figure 4A and 4B).

Bottom Line:
Three minor postoperative complications were encountered; a long-term pulmonary complication was seen in one case after reintervention and was successfully resolved after 10 days.Surgery was successful in the other 5 cases, where solid arthrodesis was achieved and no significant curve progression was observed at follow-up.In case of mild spinal deformities in arthrogryposis, brace treatment should be attempted, the evolution of the curves being unpredictable; however, when the curve exceeds 40° and presents with marked hyperkyphosis, hyperlordosis or pelvic obliquity, surgery should not be delayed.

Background: The reported incidence of scoliosis in arthrogryposis varies from 30% to 67% and, in most cases, the curves progress rapidly and become stiff from early age.The authors report six cases of scoliosis in arthrogryposis to assess the role of surgical treatment.

Methods: Six cases (3 males, 3 females; mean age at surgery 13.2 years) with arthrogryposis multiplex congenita associated with the characteristic amyoplasia were reviewed: they were operated on for scoliosis at the authors' Spine Surgery Department between 1987 and 2008.Surgery was performed using the Harrington-Luque instrumentation (2 cases), the Luque system (1), a hybrid segmental technique with hooks and screws (1) and spinal anchoring with pedicle screws (2).

Results: The patients were clinically and radiologically reviewed at a mean follow-up of 4.2 years, ± 2.7 (range, 1 to 9 years). Three minor postoperative complications were encountered; a long-term pulmonary complication was seen in one case after reintervention and was successfully resolved after 10 days. Surgery was successful in the other 5 cases, where solid arthrodesis was achieved and no significant curve progression was observed at follow-up.

Conclusions: The experience acquired with the present case series leads the authors to assert that prompt action should be taken when treating such aggressive forms of scoliosis. In case of mild spinal deformities in arthrogryposis, brace treatment should be attempted, the evolution of the curves being unpredictable; however, when the curve exceeds 40° and presents with marked hyperkyphosis, hyperlordosis or pelvic obliquity, surgery should not be delayed.